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Peripartum cardiomyopathy: experience at a tertiary care center in Puerto Rico.

Peripartum cardiomyopathy (PPCM) is an unusual cause of heart failure that is unique to pregnant women of all reproductive ages. It is a separate entity from other known existing cardiomyopathies, and it has a poorly understood pathophysiology. PPCM occurs during late pregnancy or early in the first months postpartum and is characterized by a reduced ejection fraction and the absence of any other identifiable cause of heart failure (1). Patients with PPCM have left ventricular systolic dysfunction with an ejection fraction below 45% (1).

Wide variations exist regarding the true incidence of PPCM, with several reports placing it between 1 in 300 pregnancies in places of high incidence, such as Haiti, and 1 in 4025 in areas in which the incidence is lower, such as the United States and other western societies (2-6). These discrepancies may reflect the differences in the incidence of PPCM between ethnic groups and geographical regions, as well as differences in study methodology. In the United States, there has been an overall increasing trend in the incidence of PPCM, which may be due to a heightened awareness of the condition, increases in the risk-factor burden of PPCM among pregnant women, or the expanded timeframe for diagnosis in the newer definition of PPCM (4).

Although the natural history of this condition has not been entirely elucidated, a dysfunction involving the autoimmune system (as well as abnormalities of hormonal function) is likely to be involved (7). In this report, we review our experience at the University District Hospital of Puerto Rico in treating patients diagnosed with PPCM.

Methods

This was a descriptive retrospective study that was completed after having been approved by the Institutional Review Board of the Medical Sciences Campus of the University of Puerto Rico. We conducted a chart review of all the patients who had been admitted with a diagnosis of peripartum cardiomyopathy (ICD-9 code 674.5) to the University District Hospital of Puerto Rico from January 2006 to December 2012. The inclusion criteria were as follows: 1) systolic dysfunction, as documented with an appropriate imaging study, 2) no previous history of heart failure, and 3) onset in the last month of gestation or the first 5 months of puerperium.

Quantitative and qualitative data were collected from medical charts. The following variables were recorded for each patient: age, the moment of clinical onset, medical history, gravidity and parity, the mode of delivery, perinatal complications, laboratory test parameters, the findings of a chest x-ray, the ejection fraction according to the echocardiographic report, the ejection fraction according to the multi-gated acquisition scan (if performed), demographic characteristics, and the levels of B-type natriuretic peptide. A statistical analysis was performed using the SPSS software program (SPSS Inc. Released 2009. PASW Statistics for Windows, Version 18.0. Chicago: SPSS Inc.). Descriptive analysis was performed to describe frequencies, proportions, minimum, maximum, means, and medians ([+ or -] standard deviations).

Results

Twelve cases that met the criteria for PPCM were identified. The mean age at diagnosis was 27 ([+ or -] 8) years (range 15-39), with 7 patients being 30 or older at the time of delivery. The majority of patients were multigravid, with a mean parity of 2.6 ([+ or -] 1.6). There were 2 cases involving multiple gestations.

Cesarean delivery (n = 9), preterm birth (n = 7), and preeclampsia (n = 6) were the most common complications observed in this population. Maternal comorbidities included pregestational diabetes (n = 2), gestational diabetes (n = 1), and systemic lupus erythematosus (n = 1). All the individuals were diagnosed (with PPCM) during the puerperium and for the first time in their lives. One maternal death (a consequence of complications related to cardiomyopathy) was documented. No neonatal deaths were recorded in our population.

A mean ejection fraction of 35% ([+ or -] 8) was noted overall. For 1 patient, the ejection fraction was obtained from a multi-gated acquisition scan because she had a poor echocardiographic window. Frequent echocardiographic findings included left atrial enlargement (n = 4), moderate-severe mitral regurgitation (n = 3), and pericardial effusion (n = 3). Electrocardiographic data revealed that sinus tachycardia was the most common (n = 5) abnormal finding. Four patients exhibited the electrocardiographic criteria for left ventricular hypertrophy. Six patients had evidence of pulmonary congestion on their chest radiographs. The most frequent hematological laboratory abnormality was anemia, which was present in 9 patients. Anemia was defined as a hemoglobin concentration of less than 10 g/dL (8). There was no evidence of renal failure in any of the patients evaluated. Brain natriuretic peptide (BNP) or N-terminal pro-brain natriuretic peptide (NT-proBNP) was elevated in all the patients in whom one or the other was measured (n = 10).

Discussion

This retrospective review accounts for 12 cases of Puerto Rican women who developed PPCM during pregnancy. To date, this is the only study describing pregnant Hispanic women in Puerto Rico with this condition.

Several clinical characteristics have been identified as risk factors for developing PPCM. It has been shown that a maternal age of 30 years or older is a risk factor for PPCM (9). Even though the mean age (27 [+ or -] 8 years) of our sample was below this threshold, it is interesting to note that 58% of the patients were older than 30 at the time of delivery. The majority of the patients (67%) were in their second or subsequent pregnancy, implying that having been pregnant at least once is an important risk factor for the development of this condition (mean parity: 2.6 [+ or -] 1.6). Similar findings have been well documented by Elkayam, et al (9). Although multiple-gestation pregnancies have been noted to be a risk factor for PPCM (9), only 2 of our patients had twin pregnancies. We noted that preeclampsia is strongly associated with the development of PPCM in our population, as the former was present in 50% of the investigated pregnancies. This correlates with previous accounts that report a strong clinical and pathophysiological association between these 2 disease entities (10).

All the patients included in this study met the definition of PPCM, including their having evidence of a left ventricular systolic dysfunction. In none of the patients examined was there evidence (from the clinical presentation, past medical history, family history, physical examination, laboratory data, or echocardiographic findings) to suggest that there were other possible causes of heart failure, including congenital heart disease, familial cardiomyopathy, severe valvular heart disease, tachycardia-induced cardiomyopathy, hypertrophic cardiomyopathy, and ischemic heart disease. The mean ejection fraction was 35 ([+ or -] 8). This is slightly above that of reports from populations of other ethnic backgrounds, which reports detail a left ventricular ejection fraction (LVEF) at diagnosis in the range of 20% to 31% (9, 11, 12). Although the majority of patients with PPCM experience a recovery of their cardiac function (9), it has been suggested that a low LVEF at presentation is associated with a poor prognosis and a lower probability of recovery of cardiac function (13). Regrettably, our patient data is limited to that contained in the hospital medical charts, which document only inpatient encounters. Other echocardiographic findings included mitral regurgitation, left atrial enlargement, and pericardial effusion, which have been previously described as possible echocardiographic findings in this patient population (14). The significance of these echocardiographic findings is still unknown. In addition, all the studied patients had normal sinus rhythms on their electrocardiograms, with only a minority of those patients having abnormalities such as sinus tachycardia (42%) or left ventricular hypertrophy (33%). None of the patients evaluated had evidence of arrhythmias, ST-T wave changes, or conduction abnormalities, which have been previously described (12). With regards to the laboratory test variables, most patients were noted to have significant abnormalities in terms of their hemoglobin levels and BNP or NT-proBNP levels. When using the WHO definition of anemia for pregnant patients (8), 9 patients (75%) were classified as having anemia (mean hemoglobin level of 10.5 [+ or -] 1.3). This is consistent with several studies that have identified an association between anemia and PPCM (15, 16). Furthermore, increased BNP or NT-proBNP levels were noted in all the patients in our study in whom those levels were measured (n = 10). This is consistent with the clinical presentation of the studied patients, who had clinical evidence of heart failure. Nevertheless, obtaining BNP or NT-proBNP levels is not required to make the diagnosis.

When evaluating obstetrical and neonatal outcomes, we noted a significant rate of preterm deliveries among the included cases (58%). Even though this rate is higher than what others have reported for their populations (25%) (9), in our population, it (the rate) could be secondary to a higher number of patients presenting with severe preeclampsia in whom early delivery was indicated. Accordingly, the cesarean delivery rate was substantial in our population (75%) and higher than what has been presented in other reports (40%) (9). This is expected in a group of pregnant women with multiple comorbidities, most of whom have already had a cesarean delivery in the past. Nevertheless, despite the complexity of managing these patients, only 1 patient died during hospitalization, and it was due to congestive heart failure secondary to PPCM. Thus, our mortality rate (8%) was noted to be lower than what has been reported in other populations (15%) (5).

Our study had several limitations. As noted previously, the data were obtained from a tertiary care center in Puerto Rico. Thus, our patient population was limited to cases that were referred from other institutions, which patients tended to be those with higher numbers of complications and poorer prognoses. This selection bias had a profound effect on obstetric outcome variables, such as preterm delivery rate and cesarean section rate. Additionally, this system limited the availability of long-term follow-up data because we did not have access to patient data beyond the hospitalization of that patient. As such, a future study evaluating long-term outcomes is needed in order to evaluate other possible clinical correlations in our population. Moreover, we recognize that the small sample size was an important limitation of this study, specifically, a retrospective study, which in turn severely limited the statistical analysis that could be performed and the strength of the associations. In addition, our study had several strengths, the primary being that it is the first study describing the clinical characteristics of Hispanic women in Puerto Rico with peripartum cardiomyopathy. Furthermore, given that the University District Hospital is the largest medical facility providing high-risk obstetrical services on the island and the only one that offers treatment for patients with PPCM, it is probably reasonable to conclude that our patient population represents the general population with PPCM on the island.

References

(1.) Sliwa K, Hilfiker-Kleiner D, Petrie MC, et al.; Heart Failure Association of the European Society of Cardiology Working Group on Peripartum Cardiomyopathy. Current state of knowledge on aetiology, diagnosis, management, and therapy of peripartum cardiomyopathy: a position statement from the Heart Failure Association of the European Society of Cardiology Working Group on peripartum cardiomyopathy. Eur J Heart Fail 2010;12:767-78.

(2.) Mielniczuk LM, Williams K, Davis DR, et al. Frequency of peripartum cardiomyopathy. Am J Cardiol 2006;97:1765-8.

(3.) Brar SS, Khan SS, Sandhu GK, et al. Incidence, mortality, and racial differences in peripartum cardiomyopathy. Am J Cardiol 2007;100:302-4.

(4.) Kolte D, Khera S, Aronow WS, et al. Temporal trends in incidence and outcomes of peripartum cardiomyopathy in the United States: a nationwide population-based study. J Am Heart Assoc 2014;3:e001056.

(5.) Fett JD, Christie LG, Carraway RD, Murphy JG. Five-year prospective study of the incidence and prognosis of peripartum cardiomyopathy at a single institution. Mayo Clin Proc 2005;80:1602-6.

(6.) Harper MA, Meyer RE, Berg CJ. Peripartum cardiomyopathy: population-based birth prevalence and 7-year mortality. Obstet Gynecol 2012;120:1013-9.

(7.) Ntusi NB, Mayosi BM. Aetiology and risk factors of peripartum cardiomyopathy: a systematic review. Int J Cardiol 2009;131:168-79.

(8.) WHO. Haemoglobin concentrations for the diagnosis of anaemia and assessment of severity. Vitamin and Mineral Nutrition Information System. Geneva, World Health Organization, 2011 (WHO/NMH/NHD/ MNM/11.1). Available at: Url: http://www.who.int/vmnis/indicators/ haemoglobin.pdf. Accessed June 20, 2014.

(9.) Elkayam U, Akhter MW, Singh H, et al. Pregnancy-associated cardiomyopathy: clinical characteristics and a comparison between early and late presentation. Circulation 2005;111:2050-5.

(10.) Bello N, Rendon IS, Arany Z. The relationship between pre-eclampsia and peripartum cardiomyopathy: a systematic review and meta-analysis. J Am Coll Cardiol 2013;62:1715-23.

(11.) Amos AM, Jaber WA, Russell SD. Improved outcomes in peripartum cardiomyopathy with contemporary. Am Heart J 2006;152:509-13.

(12.) Goland S, Modi K, Bitar F, et al. Clinical profile and predictors of complications in peripartum cardiomyopathy. J Card Fail 2009;15:645-50.

(13.) Goland S, Bitar F, Modi K, et al. Evaluation of the clinical relevance of baseline left ventricular ejection fraction as a predictor of recovery or persistence of severe dysfunction in women in the United States with peripartum cardiomyopathy. J Card Fail 2011;17:426-30.

(14.) Lampert MB, Lang RM. Peripartum cardiomyopathy. Am Heart J 1995;130:860-70.

(15.) Gunderson EP, Croen LA, Chiang V, Yoshida CK, Walton D, Go AS. Epidemiology of peripartum cardiomyopathy: incidence, predictors, and outcomes. Obstet Gynecol 2011;118:583-91.

(16.) Kao DP, Hsich E, Lindenfeld J. Characteristics, adverse events, and racial differences among delivering mothers with peripartum cardiomyopathy. JACC Heart Fail 2013;1:409-16.

Anthony D. Osterman-Pla, MD *; Ronald Lopez-Cepero, MD ([dagger]); Luis Jimenez, MD *; Josefina Romaguera, MD, MPH ([dagger]); Juan Aranda, MD *

* Department of Medicine; ([dagger]) Obstetrics and Gynecology of the University of Puerto Rico School of Medicine, San Juan, PR

The authors have no conflicts of interest to disclose.

Address correspondence to: Anthony D. Osterman-Pla, MD, University of Puerto Rico Medical Sciences Campus, Department of Medicine, PO Box 365067, San Juan, PR 00936. Email: anthony.osterman@upr.edu

Caption: Figure 1. Maternal characteristics of patients with PPCM * (n = 12)
Table 1. Clinical data on patients with peripartum cardiomyopathy

Mean age at diagnosis (years)    27 [+ or -] 8
Mean ejection fraction (%)       35% [+ or -] 8
Mean frequency of chest X-ray    1.7 [+ or -] 0.8
  abnormalities
Mean hemoglobin level (g/dL)     10.5 [+ or -] 1.3
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Author:Osterman-Pla, Anthony D.; Lopez-Cepero, Ronald; Jimenez, Luis; Romaguera, Josefina; Aranda, Juan
Publication:Puerto Rico Health Sciences Journal
Article Type:Report
Date:Dec 1, 2016
Words:2341
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